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He Li Fang
Hematology
About me
Loudi Central Hospital, Department of Hematology, Attending Physician.
Proficient in diseases
Specializes in the diagnosis and treatment of common diseases in hematology.
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Is Mediterranean anemia leukemia?
Thalassemia, formerly known as Mediterranean anemia or oceanic anemia, is a hereditary hemolytic anemia caused by mutations or deletions in globin genes, leading to insufficient synthesis of globin peptide chains. Those who lack beta chains are referred to as having beta-thalassemia, and those who lack alpha chains are known as having alpha-thalassemia. Clinically, it is classified into mild, intermediate, and severe forms based on the severity of anemia. The disease is widespread in many regions of the world, including the Mediterranean, the Middle East, Africa, Southeast Asia, and southern China. In China, it is more commonly found in Guangxi, Guangdong, Sichuan, Hong Kong, northern Taiwan, Yunnan, Guizhou, Hainan, Fujian, Hunan, and Hubei, and less commonly in the north. Thalassemia is fundamentally defined not as leukemia, but as a genetic disease.
Can a fetus with thalassemia be kept?
Thalassemia, commonly referred to as "Mediterranean anemia," is a hereditary hemolytic anemia caused by mutations or deletions in globin genes, leading to insufficient synthesis of globin peptide chains. Cases with a deficiency in the globin chain are called thalassemia. Regarding whether a thalassemia fetus should be carried to term, one must first consider the maternal family history, including any history of stillbirths, occurrences of hydrops fetalis (swelling in infants), or cases of severe thalassemia in children, as well as instances where both parents are carriers, marking a high-risk pregnancy. In such cases, strict prenatal diagnosis is required. Prenatal diagnosis includes taking samples of fetal chorion, amniotic fluid, and umbilical cord blood for genetic analysis. If severe thalassemia or Hemoglobin Barts Hydrops Fetalis Syndrome is detected in the fetus, the pregnancy should be terminated immediately. Therefore, it is recommended to terminate pregnancies where the fetus is found to have severe thalassemia or Hemoglobin Barts Hydrops Fetalis Syndrome.
Treatment of Iron Deficiency Anemia
The treatment principles for iron deficiency anemia mainly involve two aspects. One is to identify the cause of the iron deficiency anemia. Once the cause is confirmed, fundamental treatment of the cause should be conducted to restore the body's normal iron stores. The second aspect is iron supplementation, which is recommended to be administered orally. The daily supplemental iron dose is approximately 150 to 200 milligrams, typically using ferrous preparations such as ferrous succinate, ferrous fumarate, ferrous gluconate, and iron dextran. It is suggested that iron supplements be taken with meals or after meals to reduce gastrointestinal irritation caused by the medication. Additionally, it is advised to avoid consuming tea, coffee, and other substances that could interfere with iron absorption during the treatment period.
The causes of iron deficiency anemia
The causes of iron deficiency anemia primarily refer to the disruption of the normal dynamic balance between iron absorption and excretion, leading to the consumption of stored iron as well as an increased need for iron and insufficient iron intake, especially in cases of chronic blood loss, resulting in a long-term negative iron balance. The causes of iron deficiency can be divided into two main aspects: insufficient iron intake and excessive iron loss. In the first aspect, decreased iron intake includes dietary insufficiency, meaning inadequate food intake, and reduced absorption, which includes decreased absorption due to lack of stomach acid and reduced absorption following gastric surgery. Excessive iron loss includes the following eight aspects: 1) gastrointestinal bleeding, which includes bleeding caused by tumors, gastrointestinal ulcers, gastritis, as well as bleeding due to parasites and hookworm infections. It also includes bleeding caused by hemorrhoids in men and arteriovenous malformations; 2) excessive menstrual bleeding; 3) frequent blood donations; 4) multiple pregnancies in women; 5) chronic intravascular hemolysis causing hemoglobinuria; 6) hereditary hemorrhagic telangiectasia; 7) primary pulmonary hemosiderosis; and 8) coagulation disorders or the use of anticoagulants leading to iron deficiency. These eight categories all result from excessive loss of iron, causing iron deficiency which leads to iron deficiency anemia.
Chronic granulocytic leukemia etiology
Chronic granulocytic leukemia is a myeloproliferative tumor originating from multipotent stem cells. It is characterized by the translocation of chromosomes 9 and 22 forming the BCR/ABL fusion gene. The Philadelphia chromosome is a characteristic change in chronic granulocytic leukemia, first discovered and named in Philadelphia in 1960. Initially, it was observed as a deletion of the long arm of the primary chromosome in dividing blood cells of patients with this leukemia. Currently, studies have shown that abnormalities in the interaction between hematopoietic progenitor cells and the stroma might be central to treating the disease. Abnormal adhesion and anchoring characteristics of progenitor cells lead to disrupted cell maturation and proliferation. Chronic granulocytic cells do not adhere to stromal cells as normal cells do, particularly lacking integrin-mediated adhesion. Additionally, the expression of the adhesion molecule lymphocyte function-associated antigen 3 is also reduced in these cells. Therefore, the progression of the disease results from clonal changes. During the transformation of chronic granulocytic leukemia to acute myeloid leukemia, there is an increased rate of genetic mutations. Changes in gene expression during the progression involve various aspects, including nucleosome sugar metabolism, bone marrow myeloid differentiation, genomic instability of cell apoptosis genes, and processes related to DNA damage repair.
How is iron deficiency anemia diagnosed?
The diagnosis of iron deficiency anemia includes two aspects: one is to establish whether there is anemia caused by iron deficiency, and the other is to clarify the cause of the iron deficiency. First, we need to carefully inquire and analyze the medical history, combined with the patient's physical examination, which can provide a series of clues for diagnosing iron deficiency anemia. A definitive diagnosis also requires laboratory evidence. Clinically, we can divide iron deficiency and iron deficiency anemia into three stages: iron deficiency, iron-deficient erythropoiesis, and iron deficiency anemia. The following describes the diagnostic criteria for iron deficiency anemia specifically. Iron deficiency anemia is characterized by a significant reduction in hemoglobin in red blood cells, presenting as microcytic hypochromic anemia. Its diagnostic basis includes three aspects: first, conforming to the diagnosis of iron deficiency and iron-deficient erythropoiesis; second, having microcytic hypochromic anemia; and third, effective treatment with iron supplements. These three factors together can confirm the diagnosis of this disease.
What tests are for thalassemia?
Thalassemia firstly requires genetic diagnosis through a series of techniques such as DNA restriction endonuclease map PCR, which identify the genotype of thalassemia. Secondly, hemoglobin electrophoresis needs to be completed. Through complete hemoglobin electrophoresis, it can discriminate between silent gene carriers and those with thalassemia, hemoglobin H disease, and hemoglobin Bart's hydrops fetalis syndrome. Additionally, a significant increase in hemoglobin A2 in overt thalassemia also has certain specificity. Thirdly, a complete bone marrow picture is necessary, which will match the bone marrow picture of hemolytic anemia. There is pronounced erythroid hyperplasia, positive iron staining, and an increase in sideroblastic erythroblasts. Fourthly, a complete blood count is needed. Different types of blood counts show different levels of hemoglobin reduction. In mild thalassemia and thalassemia traits, hemoglobin is mostly normal or mildly decreased. In severe thalassemia, hemoglobin is generally below 50 grams per liter, indicating a severe anemic state. Blood smears might show anisocytosis, poikilocytosis, and target cells. It is common to see nucleated erythrocytes and reticulocytes significantly increased, which matches the signs of hemolytic anemia. Fifth, iron metabolism testing needs to be completed. By improving iron metabolism testing, it serves as a discriminant for silent gene carriers, as well as those with thalassemia traits and patients with mild thalassemia based on serum iron, iron saturation, and serum ferritin concentration. Sixth, X-ray examination needs to be completed. Severe thalassemia features typical hair-on-end changes, visible as vertical striations between the trabeculae of the cortical bone in the skull X-rays, resembling upright hair and rays of sunlight.
The key to curing iron deficiency anemia
Iron deficiency anemia, a common and frequently occurring disease in the department of hematology, is not difficult to diagnose and treat. The key to treatment lies in addressing the cause of the iron deficiency, and it is essential to eliminate the underlying causes as much as possible. Using iron supplements alone can only restore blood counts; neglecting the primary disease will not lead to a complete cure of the anemia and can easily lead to recurrence. Treatment consists of two phases: first, the supplementation with iron supplements. After taking iron supplements for three to four days, an increase in reticulocytes will occur, reaching a peak around seven days, and hemoglobin will significantly rise after two weeks of medication, returning to normal levels after one to two months. However, after hemoglobin levels normalize, some patients may stop taking the medication. It is important to note that continuing to take iron supplements for three months is necessary to replenish the body's iron stores completely.
Characteristics of iron deficiency anemia blood picture
The clinical manifestations of iron deficiency anemia are due to the specific characteristics of anemia caused by iron deficiency and the underlying disease causing the iron deficiency. Its hematological characteristics present as typical microcytic hypochromic anemia. What does this concept mean? It refers to the values in a complete blood count where MCV is less than 80 femtoliters, MCH is less than 27 picograms, and MCHC is less than 30%. MCV refers to the average red blood cell volume, MCH refers to the mean corpuscular hemoglobin concentration, and MCHC refers to the mean corpuscular hemoglobin concentration of red blood cells. The extent of changes in red blood cell indices correlates with the duration and severity of anemia. The blood smear typically shows pale-stained red blood cells with an enlarged central pallor and varying sizes. Platelet count often tends to be high when there is concurrent bleeding and generally tends to be low in infants and children. White blood cell count is usually normal or slightly reduced, with normal differential. These are the hematological characteristics of iron deficiency anemia.
Chronic Granulocytic Leukemia Classification
Chronic granulocytic leukemia is a myeloproliferative tumor originating from pluripotent stem cells, characterized by the presence of the Philadelphia chromosome or changes in the BCR/ABL fusion gene. Chronic granulocytic leukemia progresses through four stages: asymptomatic, chronic, accelerated, and blast crisis phases. Most patients are diagnosed after the onset of symptoms. Only a very few patients are diagnosed through routine physical examinations or other reasons when blood abnormalities are discovered. The earliest symptoms experienced during the chronic phase of the illness typically include fatigue, dizziness, and abdominal discomfort. The accelerated phase is a transitional stage before the blast crisis, marking a turning point where the disease worsens. It is often difficult to distinctly separate the accelerated phase from the blast crisis, and about 20%-25% of patients may enter the blast crisis phase directly without passing through the accelerated phase.